Pulmonary Vasculitis as the First Manifestation of Rheumatoid Arthritis.

SESSION TYPE: Miscellaneous Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PMINTRODUCTION: Lung disease occurs commonly in rheumatoid arthritis (RA) and is associated with significant morbidity and mortality. Recently, Fischer and colleagues demonstrated that anti-cyclic citrullinated peptide (anti-CCP) positive individuals with airways or interstitial lung disease may represent a "pre-articular" RA phenotype.CASE PRESENTATION: A 61-year-old Caucasian man, with a 7.5 pack-year smoking history, seasonal allergies, gastroesophageal reflux disease, obstructive sleep apnea, and dyslipidemia presented with pleuritic chest pain and dry cough of five months duration. There was no improvement with two courses of oral antibiotics. There was no evidence of sinusitis, arthralgias, inflammatory arthritis, rash or other symptoms of connective tissue disease. Physical examination was only notable for crackles at the right lung base. His musculoskeletal exam was normal. Cardiopulmonary testing revealed a normal nuclear cardiac stress test, a negative ventilation/perfusion scan and normal pulmonary physiology. Thoracic high-resolution computed tomography (HRCT) images revealed multiple nodules and thick-walled cavities predominantly in the right lung (Figure 1). Latent tuberculosis assessment was negative. Autoimmune serologies demonstrated a high-positive anti-CCP, anti-Ro (SSA) and a weakly positive RF. C-ANCA, P-ANCA, anti-myeloperoxidase, anti-proteinase-3, anti-nuclear and anti-glomerular basement membrane autoantibodies were negative. Surgical lung biopsy revealed necrotizing granulomatous inflammation with geographic necrosis, vasculitis and lymphocytic pleuritis. The patient was started on oral corticosteroids with rapid clinical improvement. Two months after lung biopsy, as corticosteroids were tapered, he developed symmetric inflammatory arthritis involving the small joints of the hands, wrists, and feet with synovitis His arthritis responded to dose escalation of corticosteroids and injectable methotrexate which was initiated and rapidly titrated to 25 mg weekly. Corticosteroids were tapered off over the subsequent 3 months, while synovitis symptoms, pleurisy, and cough remained quiescent. Follow-up HRCT has demonstrated that the pulmonary cavitary nodularity is improving.DISCUSSION: In this report we demonstrate that pulmonary vasculitis may be a presenting feature of RA. Although vasculitis is a well recognized extra-articular manifestation of RA, it is usually considered to be associated with long-standing, severe, erosive, nodular, and sero-positive disease. RA-vasculitis may manifest with pyoderma gangrenosum, mononeuritis multiplex, or pulmonary vasculitis. Our findings highlight that severe pulmonary vasculitis may also occur as the first clinical feature of RA.CONCLUSIONS: A wide spectrum of lung disease, including pulmonary vasculitis, may be the presenting manifestation of RA.1) Genta M.S, Genta R.M, Gabay C. Systemic Rheumatoid Vasculitis: a review. Semin Arthritis Rheum 2006. 36:88-98DISCLOSURE: The following authors have nothing to disclose: Salvador de la Torre Carazo, Olga Tourin, Daniel Smith, Fischer AryehNo Product/Research Disclosure InformationHospital 12 de Octubre, Madrid, Spain.